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Varicella zoster virus triggers the immunopathology of giant cell arteritis #MMPMID27224742
Gilden D; Nagel MA
Curr Opin Rheumatol 2016[Jul]; 28 (4): 376-82 PMID27224742show ga
Purpose of review: Giant cell arteritis (GCA) is a severe form of vasculitis in the elderly. The recent discovery of varicella zoster virus (VZV) in the temporal arteries (TA) and adjacent skeletal muscle of patients with GCA, and the rationale and strategy for antiviral and corticosteroid treatment for GCA are reviewed. Recent findings: The clinical features of GCA include excruciating headache/head pain, often with scalp tenderness, a nodular TA and decreased TA pulsations. Jaw claudication, night sweats, fever, malaise and a history of polymyalgia rheumatica (aching and stiffness of large muscles primarily in the shoulder girdle, upper back and pelvis without objective signs of weakness) are common. ESR and CRP are usually elevated. Diagnosis is confirmed by TA biopsy which reveals vessel wall damage and inflammation, with multinucleated giant cells and/or epithelioid macrophages. Skip lesions are common. Importantly, TA biopsies are pathologically negative in many clinically suspect cases. This review highlights recent virological findings in TAs from patients with pathologically-verified GCA and in TAs from patients who manifest clinical and laboratory features of GCA, but whose TA biopsies (Bx) are pathologically negative for GCA (Bx-negative GCA). Virological analysis revealed that VZV is present in most GCA-positive and GCA-negative TA biopsies, mostly in skip areas that correlate with adjacent GCA pathology. Summary: The presence of VZV in Bx-positive and Bx-negative GCA TAs indicates that VZV triggers the immunopathology of GCA. However, the presence of VZV in about 20% of TA biopsies from non-GCA post-mortem controls also suggests that VZV alone is not sufficient to produce disease. Treatment trials should be performed to determine if antiviral agents confer additional benefits to corticosteroids in both Bx-positive and Bx-negative GCA patients. These studies should also examine whether oral antiviral agents and corticosteroids are as effective as intravenous acyclovir and corticosteroids. Appropriate dosage and duration of treatment also remain to be determined.